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Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension.
Circ Heart Fail. 2013 Sep 01; 6(5):953-63.CH

Abstract

BACKGROUND

Systemic sclerosis–associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis–related pulmonary hypertension with interstitial lung disease imposes a greater pulmonary vascular load than IPAH and leads to worse RV contractile function.

METHODS AND RESULTS

We analyzed pulmonary artery pressures and mean flow in 282 patients with pulmonary hypertension (166 SScPAH, 49 systemic sclerosis–related pulmonary hypertension with interstitial lung disease, and 67 IPAH). An inverse relation between pulmonary resistance and compliance was similar for all 3 groups, with a near constant resistance×compliance product. RV pressure–volume loops were measured in a subset, IPAH (n=5) and SScPAH (n=7), as well as SSc without PH (n=7) to derive contractile indexes (end-systolic elastance [Ees] and preload recruitable stroke work [Msw]), measures of RV load (arterial elastance [Ea]), and RV pulmonary artery coupling (Ees/Ea). RV afterload was similar in SScPAH and IPAH (pulmonary vascular resistance=7.0±4.5 versus 7.9±4.3 Wood units; Ea=0.9±0.4 versus 1.2±0.5 mm Hg/mL; pulmonary arterial compliance=2.4±1.5 versus 1.7±1.1 mL/mm Hg; P>0.3 for each). Although SScPAH did not have greater vascular stiffening compared with IPAH, RV contractility was more depressed (Ees=0.8±0.3 versus 2.3±1.1, P<0.01; Msw=21±11 versus 45±16, P=0.01), with differential RV-PA uncoupling (Ees/Ea=1.0±0.5 versus 2.1±1.0; P=0.03). This ratio was higher in SSc without PH (Ees/Ea=2.3±1.2; P=0.02 versus SScPAH).

CONCLUSIONS

RV dysfunction is worse in SScPAH compared with IPAH at similar afterload, and may be because of intrinsic systolic function rather than enhanced pulmonary vascular resistive and pulsatile loading.

Authors

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Pub Type(s)

Journal Article
Multicenter Study
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

23797369

Citation

Tedford, Ryan J., et al. "Right Ventricular Dysfunction in Systemic Sclerosis-associated Pulmonary Arterial Hypertension." Circulation. Heart Failure, vol. 6, no. 5, 2013, pp. 953-63.
Tedford RJ, Mudd JO, Girgis RE, et al. Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. Circ Heart Fail. 2013;6(5):953-63.
Tedford, R. J., Mudd, J. O., Girgis, R. E., Mathai, S. C., Zaiman, A. L., Housten-Harris, T., Boyce, D., Kelemen, B. W., Bacher, A. C., Shah, A. A., Hummers, L. K., Wigley, F. M., Russell, S. D., Saggar, R., Saggar, R., Maughan, W. L., Hassoun, P. M., & Kass, D. A. (2013). Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. Circulation. Heart Failure, 6(5), 953-63.
Tedford RJ, et al. Right Ventricular Dysfunction in Systemic Sclerosis-associated Pulmonary Arterial Hypertension. Circ Heart Fail. 2013 Sep 1;6(5):953-63. PubMed PMID: 23797369.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. AU - Tedford,Ryan J, AU - Mudd,James O, AU - Girgis,Reda E, AU - Mathai,Stephen C, AU - Zaiman,Ari L, AU - Housten-Harris,Traci, AU - Boyce,Danielle, AU - Kelemen,Benjamin W, AU - Bacher,Anita C, AU - Shah,Ami A, AU - Hummers,Laura K, AU - Wigley,Fredrick M, AU - Russell,Stuart D, AU - Saggar,Rajeev, AU - Saggar,Rajan, AU - Maughan,W Lowell, AU - Hassoun,Paul M, AU - Kass,David A, PY - 2013/6/26/entrez PY - 2013/6/26/pubmed PY - 2013/11/19/medline SP - 953 EP - 63 JF - Circulation. Heart failure JO - Circ Heart Fail VL - 6 IS - 5 N2 - BACKGROUND: Systemic sclerosis–associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis–related pulmonary hypertension with interstitial lung disease imposes a greater pulmonary vascular load than IPAH and leads to worse RV contractile function. METHODS AND RESULTS: We analyzed pulmonary artery pressures and mean flow in 282 patients with pulmonary hypertension (166 SScPAH, 49 systemic sclerosis–related pulmonary hypertension with interstitial lung disease, and 67 IPAH). An inverse relation between pulmonary resistance and compliance was similar for all 3 groups, with a near constant resistance×compliance product. RV pressure–volume loops were measured in a subset, IPAH (n=5) and SScPAH (n=7), as well as SSc without PH (n=7) to derive contractile indexes (end-systolic elastance [Ees] and preload recruitable stroke work [Msw]), measures of RV load (arterial elastance [Ea]), and RV pulmonary artery coupling (Ees/Ea). RV afterload was similar in SScPAH and IPAH (pulmonary vascular resistance=7.0±4.5 versus 7.9±4.3 Wood units; Ea=0.9±0.4 versus 1.2±0.5 mm Hg/mL; pulmonary arterial compliance=2.4±1.5 versus 1.7±1.1 mL/mm Hg; P>0.3 for each). Although SScPAH did not have greater vascular stiffening compared with IPAH, RV contractility was more depressed (Ees=0.8±0.3 versus 2.3±1.1, P<0.01; Msw=21±11 versus 45±16, P=0.01), with differential RV-PA uncoupling (Ees/Ea=1.0±0.5 versus 2.1±1.0; P=0.03). This ratio was higher in SSc without PH (Ees/Ea=2.3±1.2; P=0.02 versus SScPAH). CONCLUSIONS: RV dysfunction is worse in SScPAH compared with IPAH at similar afterload, and may be because of intrinsic systolic function rather than enhanced pulmonary vascular resistive and pulsatile loading. SN - 1941-3297 UR - https://www.unboundmedicine.com/medline/citation/23797369/Right_ventricular_dysfunction_in_systemic_sclerosis_associated_pulmonary_arterial_hypertension_ L2 - http://www.ahajournals.org/doi/full/10.1161/CIRCHEARTFAILURE.112.000008?url_ver=Z39.88-2003&amp;rfr_id=ori:rid:crossref.org&amp;rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -